Interstitial lung abnormalities, when present in members of undiagnosed family members recruited on the basis of familial interstitial pneumonia, or in undiagnosed research participants, have been associated with a syndrome that includes distinct sets of imaging abnormalities, restrictive physiological and exercise impairments, and an increased prevalence of histopathological findings, and genetic predictors, that have been noted in patients with idiopathic pulmonary fibrosis. Recent longitudinal studies have demonstrated that qualitative and quantitative assessments of interstitial abnormalities are associated with accelerated lung function decline, an increased rate of clinical diagnoses of interstitial lung disease and an increased rate of mortality. In this perspective, in addition to reviewing the prior information, four major efforts that could help the field of early pulmonary fibrosis detection move forward are discussed. These efforts include: (1) developing standards for characterising and reporting imaging findings from patients with existing CTs; (2) developing consensus statements on when undiagnosed and asymptomatic imaging abnormalities should be considered a disease; (3) identifying populations for which screening efforts might be beneficial; and (4) considering approaches to developing effective secondary prevention trials.
- interstitial fibrosis
- idiopathic pulmonary fibrosis
- clinical epidemiology
- imaging/CT MRI etc
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Contributors GMH was the sole author of this publication.
Funding GMH is supported by NIH grants: R01 HL111024, R01 HL135142 and RO1 HL130974.
Competing interests GMH has been receiving funding from the National Institutes of Health and has performed consulting for the Gerson Lehrman Group, Medna, Genentech, Boehringer-Ingelheim and Mitsubishi Chemical.
Provenance and peer review Commissioned; externally peer reviewed.
Patient consent for publication Not required.
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